Objective: To determine styles of coping, that is personal ways of dealing with problems, and social support, or support from the social environment, in a cohort of adults with congenital heart disease. Methods: We subjected 362 patients with congenital heart disease, aged from 20 to 46 years, belonging to five diagnostic groups, to extensive medical and psychological examination from 20 to 33 years after their first open heart surgical procedure. During psychological examination, 342 patients filled in questionnaires concerning styles of coping, specifically the Utrecht Coping List, and social support, using the Social Support List. Results: Overall, styles of coping in the total sample are comparable to those of peers in the general population, except for lower active problem solving, which can be attributed to female patients. Males with congenital heart disease showed more favourable styles of coping compared to their peers, such as higher seeking of social support, lower passive reaction patterns, and lower expression of negative emotions. Compared to the reference group, the total cohort of patients reported to receive less social support, but also to experience less discrepancies between desired and received social support, indicating feelings of independence in these adults. Females with congenital heart disease were found to seek and receive more social support compared to their male counterparts. Conclusion: Overall, few differences in styles of coping were found between the patients and their reference groups. Perceived social support in the sample of patients was favourable.

Purpose: To assess levels of distress, the marital relationship, and styles of coping of parents of children with congenital heart disease, to evaluate any change in these parameters following elective cardiac surgery for their child, and to compare these parents with parents of children undergoing another form of hospital treatment, and with parents of healthy children. Design: A prospective study in which parents were assessed the day before the surgical procedure being undergone by their child, and 12 months afterwards. Participants: We assessed three groups of parents of 75 children, aged from birth to 16.9 years. The first was a group whose children were undergoing surgery because of congenital heart disease, the second was a group whose children were undergoing transplantation of bone marrow, and the third was a group whose children were healthy. Measures used for assessment included the General Health Questionnaire, the Dyadic adjustment scale, and the Utrecht coping list. Results: Parents in both groups of children undergoing surgery had significantly higher rates of distress prior to the surgical procedures than did the parents of the healthy children, but within those whose children were undergoing cardiac surgery, there were no differences between parents of children with cyanotic and acyanotic lesions. Following treatment, there was a significant reduction in the levels of distress in both groups whose children had undergone surgery. There were few differences between any of the groups on the other parameters, and the evaluated indexes showed stability over time. Conclusion: Despite elevated levels of psychological distress prior to surgical procedures, which had fallen after one year, the stability of other parameters of parental functioning over time suggests that the surgical interventions are of less significance than either factors attributable to the presence of chronic illness, or the individual characteristics of the parents.

Background: Creation of an extracardiac cavopulmonary connection has been proposed as a superior alternative to the lateral intracardiac tunnel for the completion of total cavopulmonary connection. Methods and results: We made a retrospective review of our experience with 125 patients undergoing a total cavopulmonary connection between June 1994 and January 2003. Our experience with the extracardiac connection for completion began in 1999. Since 1994, we have constructed an intracardiac tunnel in 50 patients, and an extracardiac connection in 75. Of the total number, 83 had undergone an earlier partial cavopulmonary connection. Additional intracardiac procedures were performed in 43 patients at time of completion, in 25 of those undergoing extracardiac completion, and in 18 of the patients having an intracardiac procedure. The mean size of the tube used for completion was 19 mm. The mean cross-clamp time for placement of the intracardiac tunnel was 77 min, with a median of 80.5 min, and a mean cardiopulmonary bypass time of 139 min, with a median of 131 min. For construction of the extracardiac connection, a mean cross-clamp time in 24 of the 75 patients was 54 min, with a median of 54 min. Mean cardiopulmonary bypass time for all the patients with an extracardiac connection was 100 min, with a median of 88 min. Reoperations were needed in 10 patients, 6 having intracardiac and 4 extracardiac procedures. Of these, 5 were early and 5 late, including one take down. None of the patients died after these interventions. Taken overall, 8 patients died, with 5 early deaths. In the multivariable analysis, cardiopulmonary bypass time of more than 120 min, atrioventricular valvar replacement, and banding of the pulmonary trunk prior to the total cavopulmonary connection, all reached statistical significance for early death, whereas only heterotaxy syndrome remained as the sole risk factor for late death. There was no significant difference in survival between the modifications used. Discussion: Whereas we could not identify any clinical superiority for the extracardiac approach in the short-term, the concept of extracardiac completion has helped to simplify the overall procedure. Longer follow-up will be required to elucidate any potential advantages.

Objectives: To estimate the incidence and timing of spontaneous closure of atrial septal defects within the oval fossa, to study the modalities for diagnosis, and predict the need for therapeutic intervention. Methods: We reviewed retrospectively the medical records of patients with isolated atrial septal defects within the oval fossa, so-called “secundum defects”, diagnosed between January 1990 and February 2003. Based on the initial echocardiographic evaluation, we divided defects into small ones measuring from 3 to 5 mm, medium ones from 5 to 8 mm, and large ones greater than 8 mm. Results: We identified 121 patients, 50 (41.3%) of whom had failed to thrive, and 14 (11.6%) had congestive heart failure. At a mean of 44.9 ± 22.1 months following diagnosis, with a range from 12 to 102 months, the defects had closed spontaneously in 31 patients (25.6%). Of 22 patients having small defects, spontaneous closure occurred in 18 (82%) at a mean age of 18.9 ± 10.2 months. Of 27 patients with defects of medium size, 12 (44%) either experienced spontaneous closure, or else the defect effectively became a patent oval foramen, at a mean age of 51.2 ± 32.2 months. Only 1 (1.4%) of the 72 patients with a defect larger than 8 mm in size underwent spontaneous closure. The defects increased in size in 8 patients (6.6%). Intervention was necessary in 76 patients (63%) at a mean age of 75.5 ± 15.2 months. Conclusion: The initial size of a defect within the oval fossa at diagnosis is the best predictor of its natural history. Some defects increase in size with growth, irrespective of their initial size.

Aim: To assess the myocardial velocities of the mitral annulus, left ventricular lateral wall, and midseptum in healthy children, and to compare these parameters with transmitral and pulmonary venous velocities. Methods and results: We examined 72 children, half being male, who had no systemic or cardiac pathologies. Their mean age was 6.73 ± 5.10 years, with a range from 0.1 to 17.75 years, and a median age of 6.71 years. Each parameter was measured twice, at end inspiration and end expiration. The tissue velocities are similar in males and females (p > 0.05). The longitudinal velocity of the heart in early diastole has a positive correlation with age (p < 0.05; midseptum velocity r = 0.57, left ventricular lateral wall velocity r = 0.56, mitral annulus velocity r = 0.56), and the tissue velocities are not influenced by respiration (p > 0.05). The myocardial velocities of different segments of the left ventricle are not correlated with the transmitral or pulmonary venous flows (p < 0.05). When age is controlled for heart rate, age mainly affects the systolic velocity of the mitral annulus and the early diastolic velocity of the midseptum in longitudinal axis, as well as the early diastolic velocity of the midseptum in transverse axis (p > 0.05 for all, r = 0.34, 0.29, 0.30 respectively). Conclusion: This study, which has determined reference values for tissue velocities in a large healthy group of children, will now set the scene for further studies in children with heart disease.

Video assisted thoracoscopic surgery for closure of the persistently patent arterial duct is an effective answer to the modern effort of seeking repair of congenital cardiac malformations with minimally invasive techniques.

Between June, 1994, and December, 2002, 150 consecutive patients with an echocardiographic diagnosis of isolated patency of the arterial duct were referred to our Institution for video assisted thoracoscopic closure. The median age at the time of operation was 45 months, with a range from 3 to 161 months, and mean weight was 18 kg, with a range from 4.2 to 73 kg. Video assisted closure was possible in 139 patients (93%). The mean operative time was 37 min, with a range from 14 to 89 min. In the remaining 11 patients (7%), seen early in our experience, we converted to a mini-thoracotomy to achieve closure. In no instance did we encounter major haemorrhage requiring blood transfusion, and there were no hospital deaths. The mean period of stay in hospital was less than 24 h in 61 patients, less than 48 h in 88 patients seen earlier in our experience, and 21 days in 1 patient because of a persistent chylothorax. Other complications included palsy of the left recurrent laryngeal nerve in 1 patient, with partial regression at 6 months follow-up.

We conclude that closure of the arterial duct by video-assisted thoroscopy combines the advantages of safe and complete extraluminal occlusion with an excellent clinical and aesthetic result, minimal postoperative pain, and short stay in hospital at low cost.

Patients with congenitally corrected transposition are at risk of right ventricular dysfunction and failure. With this in mind, we examined 13 patients with congenitally corrected transposition, 7 not having undergone surgery, and 6 after physiological repair, comparing them with 6 healthy subjects matched for age and sex, using cardiac magnetic resonance imaging, at rest and during dobutamine stress, in order to determine regional and global right ventricular response to stress.

At rest, the patients had significantly decreased overall wall motion compared to their healthy peers (7.2 ± 0.5, versus 9.8 ± 0.4 mm). During infusion of dobutamine, overall wall motion increased to 12.8 ± 0.4 mm in the healthy subjects, versus 8.8 ± 1.0 mm in patients. At the regional level, significant differences in mural motion were found between patients and controls in the anterior (9.5 ± 1.1, versus 13.2 ± 0.6 mm), posterior (10.2 ± 1.6, versus 13.2 ± 0.8 mm), and septal segments (5.0 ± 0.8, versus 11.2 ± 0.6 mm).

At rest, overall mural thickening in patients was similar to that of controls, but significantly less in patients during stress. During dobutamine stress, patients showed significantly less regional wall thickening than controls, particularly in the septal (2.7 ± 0.6, versus 6.0 ± 0.4 mm, respectively) and in the anterior segments (4.2 ± 0.6, versus 7.8 ± 0.6 mm, respectively). Right ventricular ejection fraction strongly correlated with mural motion and thickening, both at rest and during stress.

Abnormal regional function in the systemic morphologically right ventricle may occur in patients with congenitally corrected transposition, which strongly correlates with right ventricular ejection fraction. Our findings support the hypothesis that, in patients with congenitally corrected transposition, ischemia of the right ventricular myocardium contributes to the development of right ventricular dysfunction.

We undertook our study in order to evaluate right ventricular function and perfusion by conventional and contrast echocardiography in adults with congenitally corrected transposition who had not undergone cardiac surgery, comparing the echocardiographic findings with those obtained using equilibrium radionuclide ventriculography and gated single-photon emission computed tomography with Technetiumc-99 m sestamibi.

We discovered severe tricuspid regurgitation in 8 patients (61%). Right ventricular ejection fraction, as calculated by nuclear medicine, had a correlation of 0.67 (p = 0.059) with area fractional shortening and 0.84 (p = 0.01) with ejection fraction calculated by the method depending on descent of the tricuspid ring. All patients with severe tricuspid regurgitation also had right ventricular dysfunction. Of these 8 patients, 7 had persistent perfusion defects, while 6 also had ischemic perfusion defects.

Echo contrast had a high sensitivity, at 91%, and also specificity and positive predictive value, both at 100%, for persistent defects, and a negative predictive value of 66% compared to methods depending on nuclear medicine. The sensitivity of contrast echocardiography for detection of ischemic defects was 66%, the specificity 100%, the positive predictive value 100%, and the negative predictive value 77% compared to the methods involving nuclear medicine.

The method depending on descent of the tricuspid ring had the highest correlation with equilibrium radionuclide ventriculography in evaluation of right ventricular function in patients with congenitally corrected transposition.

We conclude that contrast echocardiography is extremely valuable when assessing right ventricular myocardial perfusion, having high sensitivity and specificity for detecting persistent defects, although sensitivity was less for detection of ischemic defects than that of gated single-photon emission computed tomography with Technetium-99 m Sestamibi.

Persistent and ischemic perfusion defects, together with chronic volume overload from tricuspid regurgitation, are the determining factors of right ventricular dysfunction.

A young child with exertional chest pain, and an electrocardiographic pattern suggesting reversible ischaemia of the anterior ventricular wall documented by Cardiolyte stress-testing, underwent cardiac catheterization and selective coronary angiography. Although the coronary arteries were entirely normal, the recirculation phase demonstrated marked dilation of the coronary sinus, with atresia of its mouth. At surgery, the patient was confirmed to have muscular atresia at the mouth of the coronary sinus, and underwent unroofing of the coronary sinus to the left atrium, with ligation of a persistent left superior caval vein. Post-operatively, the patient continued to have persistent chest pain, albeit without inducible ischaemia on stress-testing.

An asymptomatic 4-year-old child was referred to our Institution with a provisional diagnosis of severe aortic valvar regurgitation in association with a ventricular septal defect. Intraoperatively, the aortic valve appeared bicuspid, and a tunnel of 7 mm diameter extending between the aorta and the left ventricle was found dividing the anterior commissure into two hemi-commissures. Repair was achieved by reconstructing the anterior commissure by direct reapproximation, and simultaneous obliteration of the aortic side of the tunnel. The aortic valvar leaflet was resuspended onto the arterial wall. This combined approach will hopefully reinforce the poorly supported aortic sinus, and may maintain aortic valvar competence over the long term. Our intraoperative diagnosis of aorto-left ventricular tunnel suggests consideration of this diagnosis when evaluating any patient with suspected aortic valvar incompetence.

The acronym PHACES summarizes the most important manifestations of a rare neurocutaneous syndrome. Specifically, “P” accounts for malformation of the brain in the region of the posterior fossa, “H” stands for haemangiomas, “A” is for arterial anomalies, and “C” is for coarctation of the aorta along with cardiac defects, “E” is for abnormalities of the eye, and “S” for clefting of the sternum, and/or a supraumbilical abdominal raphe. Our objective is to introduce the syndrome to paediatric cardiologists. Our patient has stenosis of the aortic arch, multiple malformations of the great vessels arising from the aortic arch, intracranial vascular abnormalities, a sternal malformation with a supraumbilical raphe, and facial haemangiomas. We stress that it is important always to consider the existence of this syndrome in all patients with facial haemangiomas.

Aortic coarctation is a recognized arteriopathy in patients with Williams’ syndrome. We present an adolescent with Williams’ syndrome who developed rapid restenosis after primary stenting of coarctation of the aorta. We believe such restenosis within the stent is due to a proliferative response of the abnormal aortic wall. Attention should be paid to the potential for restenosis during follow-up after stenting of aortic coarctation in patients with Williams’ syndrome.

A 14-year-old female patient underwent surgical treatment of multiple atrial septal defects associated with unroofed coronary sinus and pulmonary valvar stenosis. One of the defects was that of the superior oval fossa and the other a large ellipsoidal defect positioned inferior to the inferior rim of the oval fossa. The patient underwent primary closure of the defects with a favorable result. To the best of our knowledge, this is the first surgical experience of an unusual atrial septal defect or the vestibular defect.

We report two more patients with the association of postaxial polydactyly and atrioventricular septal defects. Additional cardiac findings, not previously described, were tetralogy of Fallot in the first case, and double orifice right atrioventricular valve in the second child.